Table 1

Supportive care intervention component delivery schedule

Intervention componentRationaleApproximate time
Visit 1Visit 2Visit 3
1.Education: disease, typical disease course, prognosis, treatment options, including mechanical ventilation, intensive care unit (ICU) admissionRare disease, patients/caregivers are often uninformed about disease course and prognosis and therefore unaware of likelihood of ICU hospitalisation40%10%5%
2.Self-management training for most common and distressing symptomsProgression escalation of incapacitating symptoms, for example, cough, dyspnoea, hypoxaemia, role of pulmonary rehabilitation and oxygen therapy30%30%25%
3.Caring for caregiverImpacts family due to rapid change in life status for previously healthy individual—CG often neglects own health20%30%25%
4.Planning for future and development of shared end of life (EOL) goalsRapid progression of disease and lack of discussion prior often leaves CG without adequate preparation for making advance care planning and EOL decisions10%30%45%